New approaches for managing antiphospholipid syndrome.

Area of Science:

• Immunology
• Hematology
• Rheumatology

Background:

• Antiphospholipid antibodies (aPL) target phospholipid-binding proteins, primarily beta(2)-glycoprotein-I.
• Persistent aPL positivity necessitates a risk-stratified approach for thrombosis prevention.

Purpose of the Study:

• To review current strategies and identify gaps in primary and secondary thrombosis prevention for aPL-positive individuals.
• To explore emerging therapeutic targets for managing antiphospholipid syndrome (APS).

Main Methods:

• Literature review of prospective, controlled studies on thrombosis prevention in aPL-positive patients.
• Analysis of current recommendations and evidence for anticoagulation and adjunctive therapies in APS.
• Examination of novel treatment modalities investigated in experimental APS models.

Main Results:

• Evidence supporting aspirin for primary prevention in aPL-positive patients without vascular involvement is lacking.
• Warfarin is recommended for secondary prevention, but optimal management and alternative anticoagulants require further investigation.
• High-intensity anticoagulation efficacy in APS with vascular involvement is not well-established.
• Catastrophic APS treatment yields high mortality despite aggressive multimodal therapy.
• Emerging therapies like hydroxychloroquine, statins, rituximab, and complement inhibitors show promise in preclinical studies.

Conclusions:

• A risk-stratified approach is essential for primary thrombosis prevention in aPL-positive individuals.
• Further research is critical to define optimal anticoagulation strategies and evaluate novel therapies for APS.
• Despite aggressive treatments, catastrophic APS remains associated with high mortality, highlighting the need for improved management.